Article(id=1292130406720102651, tenantId=1146029695717560320, journalId=1246415772164075586, issueId=1292130360968630762, articleNumber=null, orderNo=null, doi=10.13699/j.cnki.1001-6821.2026.06.013, pmid=null, cstr=null, oa=null, hot=null, price=null, onlineType=0, articleFormat=0, articleType=null, articleTypeStr=null, receivedDate=1767715200000, receivedDateStr=2026-01-07, revisedDate=null, revisedDateStr=null, acceptedDate=null, acceptedDateStr=null, onlineDate=1785997495583, onlineDateStr=2026-08-06, pubDate=1774627200000, pubDateStr=2026-03-28, doiRegisterDate=null, doiRegisterDateStr=null, onlineIssueDate=1785997495583, onlineIssueDateStr=2026-08-06, onlineJustAcceptDate=null, onlineJustAcceptDateStr=null, onlineFirstDate=null, onlineFirstDateStr=null, sourceXml=null, magXml=null, createTime=1785997495583, creator=13701087609, updateTime=1785997495583, updator=13701087609, issue=Issue{id=1292130360968630762, tenantId=1146029695717560320, journalId=1246415772164075586, year='2026', volume='42', issue='6', pageStart='751', pageEnd='900', issueExtLink='null', onlineDate='null', pubDate='1774627200000', pubDateStr='2026-03-28', beforeIssueId=null, nextIssueId=null, price=null, status=1, issueComplete=1, articleOrder=1, issueType=-1, specialIssue=null, createTime=1785997484675, creator='13701087609', updateTime=1786014452071, updator='13701087609', preIssue=null, nextIssue=null, articleTotal=null, ext={EN=IssueExt(id=1292201527440068743, tenantId=1146029695717560320, journalId=1246415772164075586, issueId=1292130360968630762, language=EN, specialIssueTitle=, coverIllustrator=null, specialIssueEditor=, specialIssueAbout=), CN=IssueExt(id=1292201527440068744, tenantId=1146029695717560320, journalId=1246415772164075586, issueId=1292130360968630762, language=CN, specialIssueTitle=, coverIllustrator=null, specialIssueEditor=, specialIssueAbout=)}, issueFiles=null, downloadFileDto=null}, startPage=835, endPage=839, ext={EN=ArticleExt(id=1292130408532041980, articleId=1292130406720102651, tenantId=1146029695717560320, journalId=1246415772164075586, language=EN, title=Phenobarbital treatment strategy for pregnant patient with Crigler-Najjar syndrome type Ⅱ, columnId=1246531412023730850, journalTitle=Chinese Journal of Clinical Pharmacology, columnName=Medication Comment, runingTitle=null, highlight=null, articleAbstract=
Objective This article aims to explore the medication strategy for the treatment of type Ⅱ Crigler-Najjar syndrome (CNS II) during pregnancy with phenobarbital, focusing on the relationship between treatment dosage, treatment duration, and gestational stage of the patient with maternal and neonatal outcomes, providing a reference for the treatment of this rare disease during pregnancy.
Methods : This study systematically searched the PubMed, Embase, Web of Science, and Cochrane Library database using the keywords “Pregnancy” and “Crigler-Najjar syndrome Ⅱ” with the logical connector “and”. The time range covered from the establishment of the database to March 2025. A total of 11 articles were screened.
Results A total of 11 patients with CNS Ⅱ during pregnancy were included. Among them, 3 cases did not receive drug treatment, and 2 cases had known total bilirubin levels, which were 7 mg·dL-1 and 8.5 mg·dL-1, respectively. Among the 3 cases, 1 newborn was healthy, 1 had jaundice that could subside on its own, and 1 had jaundice that required ursodeoxycholic acid treatment. The total bilirubin levels of the remaining 8 cases were between 6.85 and 21.5 mg·dL-1, and all received phenobarbital treatment, with a dosage of 25-60 mg·d-1. After treatment, 1 out of 8 newborns was completely healthy, 4 had varying degrees of jaundice, and all received 1-3 days of phototherapy and recovered. Follow-up was conducted for 6 months to 11 years for 3 of the infants, and no neurological damage was observed. During the treatment process, 5 patients received bilirubin level monitoring, once a week in the first month and once a month thereafter.
Conclusion The current evidence suggests that the maternal bilirubin level may be one of the key factors influencing the neonatal outcome. When the bilirubin level remains above 8-10 mg·dL-1, some studies recommend considering drug intervention under multidisciplinary assessment. However, due to the extremely small sample size and the lack of controlled studies, the causal relationship between the use of phenobarbital and maternal and infant outcomes cannot be clearly determined. The clinical benefits of phenobarbital use still need to be verified by more research. After thorough assessment by the treatment team, if phenobarbital intervention is chosen, it can be started in the early stage of pregnancy and continued until delivery, with a recommended dose of less than 60 mg·d-1. During the treatment period, the maternal total bilirubin concentration should be closely monitored, once a week in the first month and once a month thereafter, to ensure the safety of medication.
, authors=Zhuo SUN, Peng-jiao AN, Bo ZHANG, Yi-li SHI, authorsList=Zhuo SUN, Peng-jiao AN, Bo ZHANG, Yi-li SHI, authorCompany=null, correspAuthors=Bo ZHANG, Yi-li SHI, authorNote=null, correspAuthorsNote=null, copyrightStatement=null, copyrightOwner=null, extLink=null, articleAbsUrl=null, sourceXml=null, magXml=null, pdfUrl=null, pdf=null, pdfFileSize=null, pdfExtLink=null, richHtmlUrl=null, mobilePdfUrl=null, reviewReport=null, pdfFirstPage=null, abstractGraph=null, abstractGraphContent=null, abstractVideo=null, citation=null, cebUrl=null, magXmlContent=null, mapNumber=null, fund=null), CN=ArticleExt(id=1292130408783700221, articleId=1292130406720102651, tenantId=1146029695717560320, journalId=1246415772164075586, language=CN, title=Crigler-Najjar综合征Ⅱ型妊娠期患者的苯巴比妥治疗策略, columnId=1246531412271194800, journalTitle=中国临床药理学杂志, columnName=用药点评, runingTitle=null, highlight=null, articleAbstract=
目的 本文旨在探讨Crigler-Najjar综合征Ⅱ型（CNS Ⅱ型）妊娠期患者应用苯巴比妥治疗的用药策略，重点分析治疗剂量、治疗周期、患者妊娠分期等与母婴结局的关系，为该罕见病合并妊娠的治疗提供参考。
方法 本研究通过系统检索PubMed、Embase、Web of Science和Cochrane Library数据库，检索词包括：“Pregnancy”和“Crigler-Najjar syndrome type Ⅱ”，时间检索范围覆盖建库至2025年3月，最终共筛选出11篇文章。
结果 共纳入11例年龄介于24~37岁的CNS Ⅱ型妊娠患者。其中3例未接受药物治疗，2例已知总胆红素水平，分别为7和8.5 mg·dL-1，3例患者的新生儿1例健康，1例出现可自行消退的黄疸，1例出现胆汁淤积需要熊去氧胆酸治疗。其余8例患者总胆红素水平在6.85~21.5 mg·dL-1之间，均接受苯巴比妥治疗，剂量为25~60 mg·day-1。治疗后，8例新生儿中1例完全健康，4例出现不同程度的黄疸，均接受1~3日光疗后恢复。对其中3例婴儿进行6个月至11年随访，未见神经功能损伤。治疗过程中，5例患者接受了胆红素水平监测，首月每周1次，之后每月1次。
结论 现有证据提示，母体胆红素水平可能是影响新生儿结局的关键因素之一。当胆红素水平持续高于8~10 mg·dL-1时，部分研究建议可在多学科评估下考虑药物干预。然而，由于样本量极小，且缺乏对照研究，尚无法明确苯巴比妥使用与母婴结局之间的因果关系，其临床获益仍需更多研究验证。如选择苯巴比妥干预，可于孕早期开始用药并至生产，剂量建议低于60 mg·day-1，且治疗期间应密切监测母血总胆红素浓度，首月每周1次，之后每月1次，以保障用药安全。
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孙卓（1993-），女，药师，主要从事神经系统罕见病药物临床研究
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